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Advances in the Diagnosis of Interstitial Lung Diseases

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A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (31 July 2023) | Viewed by 8595

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Special Issue Editor

Prof. Dr. Martina Bonifazi

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Guest Editor

Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy
Interests: interstitial lung diseases; interventional pulmonology; pleural diseases; chronic obstructive lung diseases; epidemiology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

The term “interstitial lung diseases” (ILDs) includes a wide spectrum of heterogenous entities with different prognoses and treatment options. Due to the recent progress in the diagnostic and therapeutic landscape of ILD management, an accurate and early diagnosis of a progressive fibrosing ILD is crucial to improve the survival and quality of life of these patients. A diagnostic work-up is, however, still a challenging process, as, according to the ATS/ERS guidelines, it requires an integrated multidisciplinary approach involving pulmonologists, radiologists, and, when classic criteria are not met, also pathologists. Medical history, physical examination, lung function tests, high-resolution computed tomography, bronchoalveolar lavage, and, in case of still inconclusive results, a lung tissue sample are key steps for diagnosis.The huge progress in the field of ILD over the recent decades have coupled with outstanding advancements in technology in interventional pulmonology and radiology, leading to the development of innovative, more accurate and less invasive diagnostic tools. For example, new sophisticated guidance systems have been developed and adopted in interventional pulmonology practice to allow limited and early parenchymal alterations, and these include navigation technology, confocal microscopy, cone-beam computed tomography and other tools. Moreover, in the field of radiology, micro-TC and sophisticated software are now available to identify specific patterns and the relative extent, and they are likely to improve diagnostic accuracy. Finally, molecular analyses to detect usual interstitial pneumonia (UIP) signatures in lung samples have been developed and studies assessing their accuracy are currently ongoing. The main goals of this Special Issue are to update and summarize the innovations in diagnostic approach of ILDs and leaders in the different fields will be invited to provide comprehensive reviews on these topics.

Prof. Dr. Martina Bonifazi
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Diagnostics is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

innovations
new diagnostic tools
guidance systems
diffuse parenchymal lung diseases
interventional pulmonology
molecular analyses
radiology

Published Papers (5 papers)

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Research

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10 pages, 729 KiB

Open AccessArticle

New 6-Minute-Walking Test Parameter—Distance/Desaturation Index (DDI) Correctly Diagnoses Short-Term Response to Immunomodulatory Therapy in Hypersensitivity Pneumonitis

by Katarzyna B. Lewandowska, Małgorzata Sobiecka, Piotr W. Boros, Małgorzata Dybowska, Inga Barańska, Małgorzata E. Jędrych, Agata Gładzka, Witold Z. Tomkowski and Monika Szturmowicz

Diagnostics 2023, 13(6), 1109; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics13061109 - 15 Mar 2023

Cited by 2 | Viewed by 1413

Abstract

The six-minute-walking test (6MWT) is an easy-to-perform, cheap and valuable tool to assess the physical performance of patients. It has been used as one of the endpoints in many clinical trials investigating treatment efficacy in pulmonary arterial hypertension and idiopathic pulmonary fibrosis. However, the utility of 6MWT in patients diagnosed with hypersensitivity pneumonitis (HP) is still under investigation. The aim of the present retrospective study was to assess the value of different 6MWT parameters, including the newly developed distance-desaturation index (DDI), to evaluate immunomodulatory treatment outcomes in HP patients. Methods: 6MWT parameters (distance, initial saturation, final saturation, desaturation, distance-saturation product (DSP), and DDI) were analyzed at baseline and after 3 to 6 months of treatment with corticosteroids alone or in combination with azathioprine. Results: 91 consecutive HP patients diagnosed and treated in a single pulmonary unit from 2005 to 2017 entered the study. There were 44 (48%) males and 52 (57%) patients with fibrotic HP (fHP). Sixty-three patients (69%) responded to treatment (responders) and 28 (31%) did not respond (non-responders). In the responders group, all parameters assessed during 6MWT significantly improved, whereas in non-responders, they worsened. Medians (95% CI) of best indices were post-treatment DDI/baseline DDI—1.67 (1.85–3.63) in responders versus 0.88 (0.7–1.73) in non-responders (p = 0.0001) and change in walking distance–51 m (36–72 m) in responders, versus 10.5 m (−61.2–27.9) in non-responders (p = 0.0056). The area under the curve (AUC) of receiver operating characteristics (ROC) for post-treatment DDI/baseline DDI was 0.74 and the optimal cut-off was 1.075, with 71% of specificity and 71% of sensitivity. Conclusions: 6MWT may be used as a tool to assess and monitor the response to immunomodulatory therapy in HP patients, especially if indices incorporating both distance and desaturation are used. Based on the present study results, we recommend 6MWD and DDI use, in addition to FVC and TL,co, to monitor treatment efficacy in patients with interstitial lung diseases. Full article

(This article belongs to the Special Issue Advances in the Diagnosis of Interstitial Lung Diseases)

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12 pages, 1040 KiB

Open AccessArticle

Bronchoalveolar Lavage Cell Count and Lymphocytosis Are the Important Discriminators between Fibrotic Hypersensitivity Pneumonitis and Idiopathic Pulmonary Fibrosis

by Małgorzata Sobiecka, Monika Szturmowicz, Katarzyna B. Lewandowska, Inga Barańska, Katarzyna Zimna, Ewa Łyżwa, Małgorzata Dybowska, Renata Langfort, Piotr Radwan-Röhrenschef, Adriana Roży and Witold Z. Tomkowski

Diagnostics 2023, 13(5), 935; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics13050935 - 01 Mar 2023

Cited by 2 | Viewed by 1715

Abstract

Background: Fibrotic hypersensitivity pneumonitis (fHP) shares many features with other fibrotic interstitial lung diseases (ILD), and as a result it can be misdiagnosed as idiopathic pulmonary fibrosis (IPF). We aimed to determine the value of bronchoalveolar lavage (BAL) total cell count (TCC) and lymphocytosis in distinguishing fHP and IPF and to evaluate the best cut-off points discriminating these two fibrotic ILD. Methods: A retrospective cohort study of fHP and IPF patients diagnosed between 2005 and 2018 was conducted. Logistic regression was used to evaluate the diagnostic utility of clinical parameters in differentiating between fHP and IPF. Based on the ROC analysis, BAL parameters were evaluated for their diagnostic performance, and optimal diagnostic cut-offs were established. Results: A total of 136 patients (65 fHP and 71 IPF) were included (mean age 54.97 ± 10.87 vs. 64.00 ± 7.18 years, respectively). BAL TCC and the percentage of lymphocytes were significantly higher in fHP compared to IPF (p < 0.001). BAL lymphocytosis >30% was found in 60% of fHP patients and none of the patients with IPF. The logistic regression revealed that younger age, never smoker status, identified exposure, lower FEV₁, higher BAL TCC and higher BAL lymphocytosis increased the probability of fibrotic HP diagnosis. The lymphocytosis >20% increased by 25 times the odds of fibrotic HP diagnosis. The optimal cut-off values to differentiate fibrotic HP from IPF were 15 × 10⁶ for TCC and 21% for BAL lymphocytosis with AUC 0.69 and 0.84, respectively. Conclusions: Increased cellularity and lymphocytosis in BAL persist despite lung fibrosis in HP patients and may be used as important discriminators between IPF and fHP. Full article

(This article belongs to the Special Issue Advances in the Diagnosis of Interstitial Lung Diseases)

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11 pages, 1232 KiB

Open AccessArticle

Factors Predictive for Immunomodulatory Therapy Response and Survival in Patients with Hypersensitivity Pneumonitis—Retrospective Cohort Analysis

by Katarzyna B. Lewandowska, Inga Barańska, Małgorzata Sobiecka, Piotr Radwan-Rohrenschef, Małgorzata Dybowska, Monika Franczuk, Adriana Roży, Agnieszka Skoczylas, Iwona Bestry, Jan Kuś, Witold Z. Tomkowski and Monika Szturmowicz

Diagnostics 2022, 12(11), 2767; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics12112767 - 12 Nov 2022

Cited by 5 | Viewed by 1239

Abstract

Hypersensitivity pneumonitis (HP) is one of the interstitial lung diseases with clearly established diagnostic criteria. Nevertheless, pharmacologic treatment recommendations are still lacking. Most specialists use steroids as first-line drugs, sometimes combined with an immunosuppressive agent. Aim: The aim of the present retrospective study was to establish predictive factors for treatment success and survival advantage in HP patients. Methods: We analyzed the short-term treatment outcome and overall survival in consecutive HP patients treated with prednisone alone or combined with azathioprine. Results: The study group consisted of 93 HP patients, 54 (58%) with fibrotic HP and 39 (42%) with non-fibrotic HP. Mean (± SD) VCmax % pred. and TL,co % pred. before treatment initiation were 81.5 (±20.8)% and 48.3 (±15.7)%, respectively. Mean relative VCmax and TL,co change after 3–6 months of therapy were 9.5 (±18.8)% and 21.4 (±35.2)%, respectively. The short-term treatment outcomes were improvement in 49 (53%) patients, stabilization in 16 (17%) patients, and progression in 28 (30%) patients. Among those with fibrotic HP, improvement was noted in 19 (35%) cases. Significant positive treatment outcome predictors were fever after antigen exposure, lymphocyte count in broncho-alveolar lavage fluid (BALF) exceeding 54%, RV/TLC > 120% pred., and ill-defined centrilobular nodules in high-resolution computed tomography (HRCT). An increased eosinophil count in BALF and fibrosis in HRCT were significant negative treatment outcome predictors. The presence of fibrosis in HRCT remained significant in a multivariate analysis. A positive response to treatment, as well as preserved baseline VCmax (% pred.) and TLC (% pred.), predicted longer survival, while fibrosis in HRCT was related to a worse prognosis. Conclusion: Immunomodulatory treatment may be effective in a significant proportion of patients with HP, including those with fibrotic changes in HRCT. Therefore, future trials are urgently needed to establish the role of immunosuppressive treatment in fibrotic HP. Full article

(This article belongs to the Special Issue Advances in the Diagnosis of Interstitial Lung Diseases)

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14 pages, 1472 KiB

Open AccessArticle

A New Method for the Assessment of Myalgia in Interstitial Lung Disease: Association with Positivity for Myositis-Specific and Myositis-Associated Antibodies

by Gianluca Sambataro, Chiara Alfia Ferrara, Carla Spadaro, Sebastiano Emanuele Torrisi, Giovanna Vignigni, Ada Vancheri, Giuseppe Muscato, Nicoletta Del Papa, Michele Colaci, Lorenzo Malatino, Stefano Palmucci, Lorenzo Cavagna, Giovanni Zanframundo, Francesco Ferro, Chiara Baldini, Domenico Sambataro and Carlo Vancheri

Diagnostics 2022, 12(5), 1139; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics12051139 - 04 May 2022

Cited by 5 | Viewed by 1566

Abstract

In this study, it was found that myositis-specific and myositis-associated antibodies (MSAs and MAAs) improved the recognition of idiopathic inflammatory myopathies (IIMs) in interstitial lung disease (ILD) patients. The objective of this study is to propose a clinical method to evaluate myalgia in respiratory settings as a possible tool for the recognition of MSA/MAA positivity in ILD patients. We prospectively enrolled 167 ILD patients with suspected myositis, of which 63 had myalgia evoked at specific points (M+ILD+). We also enrolled in a 174 patients with only myalgia (M+ILD-) in a rheumatological setting. The patients were assessed jointly by rheumatologists and pulmonologists and were tested for autoantibodies. M+ILD+ patients were positive for at least one MAA/MSA in 68.3% of cases, as were M-ILD+ patients in 48.1% of cases and M+ILD- patients in 17.2% of cases (p = 0.01 and <0.0001, respectively). A diagnosis of IIM was made in 39.7% of M+ILD+ patients and in 23.1% of the M-ILD+ group (p = 0.02). Myalgia was significantly associated with positivity for MSA/MAAs in ILD patients (p = 0.01, X²: 6.47). In conclusion, myalgia in ILD patients with suspected myositis is associated with MSA/MAA positivity, and could support a diagnosis of IIM. A significant proportion of M+ILD- patients also had MSA/MAA positivity, a phenomenon warranting further study to evaluate its clinical meaning. Full article

(This article belongs to the Special Issue Advances in the Diagnosis of Interstitial Lung Diseases)

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Other

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9 pages, 2239 KiB

Open AccessCase Report

Acute Eosinophilic Pneumonia Complicated with Venous Thromboembolic Disease—Diagnostic and Therapeutic Considerations

by Ewa Jankowska, Iwona Bartoszuk, Katarzyna Lewandowska, Małgorzata Dybowska, Lucyna Opoka, Witold Tomkowski and Monika Szturmowicz

Diagnostics 2022, 12(6), 1425; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics12061425 - 09 Jun 2022

Viewed by 2013

Abstract

Acute Eosinophilic Pneumonia (AEP) is a rare idiopathic disease caused by an accumulation of eosinophils in the pulmonary alveoli and interstitial tissue of the lungs. The onset of symptoms is acute; some patients develop respiratory failure. The diagnosis is based on clinical symptoms, diffuse interstitial infiltrates in the lungs on imaging studies, and eosinophilia exceeding 25% on bronchoalveolar lavage or pleural fluid smear. Smokers are primarily at increased risk for the disease. We present a case of venous thromboembolic disease (VTE) that developed in the course of AEP in a previously healthy male smoker. Complete remission of the disease was achieved with anticoagulation therapy combined with a low dose of steroids. Surprisingly, further diagnostics revealed the presence of thrombophilia: antithrombin (AT) deficiency and increased homocysteine level. According to our knowledge, this is the first case of VTE diagnosed in the course of AEP combined with thrombophilia. Full article

(This article belongs to the Special Issue Advances in the Diagnosis of Interstitial Lung Diseases)

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